We report a case of lymphangiosarcoma in an 81-year-old male patient. Two months prior to his first visit, a 4-cm-sized, slightly indistinct, pale reddish-brown, edematous, infiltrating erythema appeared on his right cheek. He visited his previous physician, who suspected granulomatous disease or a deep cutaneous fungal infection, and performed a fungal culture and skin biopsy. Fungal culture was negative.
Histopathological findings revealed the formation of a slit-like vascular structure by vascular endothelial cells and their proliferation. Few erythrocytes were found inside and outside the blood vessels, and immunostaining was positive for D2-40, leading to the diagnosis of angiosarcoma with lymphangiogenic differentiation. After chemoradiotherapy (a total of 58 Gy of radiation and weekly paclitaxel (80 mg/m2)), the skin rash flattened and showed a tendency to fade. Angiosarcomas can be classified into two types based on their clinical and histological characteristics : hemangiosarcoma (angiosarcoma in the narrow sense) and lymphangiosarcoma.
Lymphangiosarcoma without chronic lymphedema is less hemorrhagic than hemangiosarcoma and tends to have an atypical clinical presentation due to the absence of nodular lesions. Similar to hemangiosarcoma, it is a disease with a poor prognosis and should be treated with caution because it is difficult to diagnose and often appears to be nonmalignant.[Skin Cancer (Japan) 2022 ; 37 : 197-202]
View full abstract